Myozenin: An α-actinin- and γ-filamin-binding protein of skeletal muscle Z lines
Myozenin: An α-actinin- and γ-filamin-binding protein of skeletal muscle Z lines
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DOI：
10.1073/pnas.98.4.1595
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发表时间：
2001-02
影响因子：
11.1
通讯作者：
F. Takada;Douglas L. Vander Woude;H. Tong;T. Thompson;S. Watkins;L. Kunkel;A. Beggs
中科院分区：
文献类型：
--
作者：
F. Takada;Douglas L. Vander Woude;H. Tong;T. Thompson;S. Watkins;L. Kunkel;A. Beggs
为了更好地了解Z系的结构和功能，我们利用α-肌动蛋白和γ-细丝蛋白的肌瘤异构体，利用酵母双杂交系统筛选了人骨骼肌相互作用蛋白的文库。在这里，我们描述了Myozenin(MYOZ)，一种主要在骨骼肌中表达的α-肌动蛋白和γ-细丝蛋白结合的Z线蛋白。Myozenin被预测为一个32 kDa的球状蛋白，其中心富含甘氨酸的区域两侧是α-螺旋区域，与任何已知基因没有很强的同源性。MYOZ基因有6个外显子，定位于人类染色体10q22.1-q22.2。Northern印迹分析表明，该转录本主要在骨骼肌中表达，在其他几个组织中的表达水平明显较低。抗肌粘菌素抗血清将骨骼肌染色成肉瘤图案，与使用α-肌动蛋白抗体所见的图案难以区分，免疫金电子显微镜证实了Z线的特异性定位。因此，myozenin是一种骨骼肌Z线蛋白，可能是肢体带状肌营养不良或其他神经肌肉疾病的一个很好的候选基因。
To better understand the structure and function of Z lines, we used sarcomeric isoforms of α-actinin and γ-filamin to screen a human skeletal muscle cDNA library for interacting proteins by using the yeast two-hybrid system. Here we describe myozenin (MYOZ), an α-actinin- and γ-filamin-binding Z line protein expressed predominantly in skeletal muscle. Myozenin is predicted to be a 32-kDa, globular protein with a central glycine-rich domain flanked by α-helical regions with no strong homologies to any known genes. The MYOZ gene has six exons and maps to human chromosome 10q22.1-q22.2. Northern blot analysis demonstrated that this transcript is expressed primarily in skeletal muscle with significantly lower levels of expression in several other tissues. Antimyozenin antisera stain skeletal muscle in a sarcomeric pattern indistinguishable from that seen by using antibodies for α-actinin, and immunogold electron microscopy confirms localization specifically to Z lines. Thus, myozenin is a skeletal muscle Z line protein that may be a good candidate gene for limb-girdle muscular dystrophy or other neuromuscular disorders.